Cases reported "Pituitary Diseases"

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1/95. Infratentorial subdural empyema, pituitary abscess, and septic cavernous sinus thrombophlebitis secondary to paranasal sinusitis: case report.

    OBJECTIVE AND IMPORTANCE: Infratentorial empyema, pituitary abscess, and septic cavernous sinus thrombophlebitis are all rare and potentially lethal conditions. The occurrence of all three in a single patient has not previously been described. We present such a case occurring in a young, otherwise healthy man. CLINICAL PRESENTATION: A 26-year-old man with a remote history of sinusitis developed rapidly progressive headache, fever, right eye pain, swelling, proptosis, and visual impairment. magnetic resonance imaging demonstrated diffuse pansinusitis, including sphenoid sinusitis, and extension of inflammation and infection into the adjacent cavernous sinuses, pituitary gland, and posterior fossa. INTERVENTION: Urgent drainage of the ethmoid and maxillary sinuses was performed; pus was not identified. The patient continued to deteriorate clinically with worsening of visual acuity. Computed tomography of the head performed the next day revealed worsening hydrocephalus and an enlarging posterior fossa subdural empyema. Urgent ventricular drainage and evacuation of the empyema was performed, and subsequently, the patient's clinical course improved. The microbiology results revealed alpha hemolytic streptococcus and coagulase-negative staphylococcus species. The patient survived but during the follow-up period had a blind right eye and pituitary insufficiency. CONCLUSION: Paranasal sinusitis can have devastating intracranial sequelae. Involvement of the adjacent pituitary gland and cavernous sinuses can result in serious neurological morbidity or mortality, and retrograde spread of infection through the basal venous system can result in subdural or parenchymal brain involvement. A high index of suspicion and aggressive medical and surgical treatment are crucial for patient survival, but the morbidity rate remains high. Our patient survived but lost anterior pituitary function and vision in his right eye.
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2/95. Rosai-Dorfman disease presenting as a pituitary tumour.

    A 45-year-old woman had pyrexia, headaches, collapse and hyponatraemia. Intracerebral abscess, bacterial meningitis and subarachnoid haemorrhage were excluded. She was given intravenous antibiotics and gradually recovered. One month later she was readmitted with diplopia, headache and vomiting. serum sodium was low (107 mmol/l) and a diagnosis of inappropriate ADH secretion was made. MRI scan showed a suprasellar tumour arising from the posterior pituitary gland. A skin rash gradually faded. serum cortisol, prolactin, gonadotrophins and thyroid hormone levels were low. A pituitary tumour was removed trans-sphenoidally, she had external pituitary radiotherapy, and replacement hydrocortisone and thyroxine. She was well for 12 months when she developed progressive weakness and numbness of both legs. Examination suggested spinal cord compression at the level of T2 where MRI scanning showed an intradural enhancing mass. This spinal tumour was removed and her neurological symptoms disappeared. Nine months after this she developed facial pain and nasal obstruction. CT scan showed tumour growth into the sphenoid sinus and nasal cavities. A right Cauldwell-Luc operation was done and residual tumour in the nasal passages was treated by fractionated external radiotherapy and prednisolone. Histological examination of the specimens from pituitary, spinal mass, and nasal sinuses showed Rosai-Dorfman disease, a rare entity characterized by histiocytic proliferation, emperipolesis (lymphophagocytosis) and lymphadenopathy. aged 48 she developed cranial diabetes insipidus. Although Rosai-Dorfman syndrome is rare, it is being reported with increasing frequency, and should be borne in mind as a possible cause of a pituitary tumour.
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3/95. pituitary gland gumma in congenital syphilis after failed maternal treatment: a case report.

    A preterm, very low birth weight infant was born to a mother with early latent syphilis who was treated 10 days and 3 days before delivery with 2.4 mU of benzathine penicillin. The infant had clinical, laboratory, and radiographic abnormalities consistent with congenital syphilis, ie, a Venereal disease research Laboratory test titer that was fourfold greater than was the maternal titer, hepatosplenomegaly, abnormal liver function tests, pneumonitis, osteochondritis of the long bones, and cerebrospinal fluid (CSF) examination showing a reactive Venereal disease research Laboratory test, pleocytosis, and elevated protein content. The infant died on the third day of life, and an autopsy revealed an evolving gumma of the anterior pituitary. immunoglobulin m immunoblotting of serum and CSF was positive, and polymerase chain reaction detected treponema pallidum dna in endotracheal aspirate and CSF. This case highlights the pathologic abnormalities observed in congenital syphilis and focuses on the rare finding of an evolving anterior pituitary gumma. Furthermore, it documents the failure of maternal syphilis treatment during the last 4 weeks of pregnancy to cure fetal infection and supports the recommendation that all infants born to mothers with syphilis treated during the last 4 weeks of pregnancy should receive penicillin therapy.
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4/95. Granulomatous hypophysitis due to Wegener's granulomatosis.

    We describe the MR image findings in a case of granulomatous hypophysitis due to Wegner's granulomatosis. A high index suspicion of hypophysitis based on imaging findings allowed successful medical management and helped avoid surgery. The MR imaging features included a thickened stalk, a diffusely and uniformly enlarged gland, a normal size or minimally enlarged sella, and enhancement of the optic chiasm.
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5/95. A case of lymphocytic infundibuloneurohypophysitis: histophathological studies.

    A 57-year-old woman presented with 2-year history of polyuria and polydipsia. Hormonal studies revealed almost normal anterior pituitary function and central diabetes insipidus. magnetic resonance imaging showed thickening of the pituitary stalk and enlargement of the neurohypophysis without high intensity of the posterior lobe on T1-weighted images, which were compatible with lymphocytic infundibuloneurohypophysitis. Transsphenoidal biopsy was done and histological examination disclosed moderate fibrosis and lymphocytic infiltration not only in the posterior pituitary, but also in the adjacent anterior pituitary part of the gland. The lymphocytes both in the anterior and posterior pituitary were mainly T cells that were positive for UCHL 1, CD 3, and CD 8. Immunofluorescence of frozen tissue detected immunecomplex deposition in small vessels and the interstitium. These findings suggested that allergic reactions may play an important role in the pathogenesis of lymphocytic infundibuloneurohypophysitis.
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6/95. Lymphocytic hypophysitis masking a suprasellar germinoma in a 12-year-old girl--a case report.

    Case history, light and electron microscopic findings of a case of a lymphocytic hypophysitis in coincidence with a suprasellar germinoma in a 12-year-old girl are reported. The girl presented with a long time case history of diabetes insipidus and subsequent panhypopituitarism. Two years after the diagnosis of diabetes insipidus magnetic resonance imaging (MRI) showed a tumorous enlargement of the sellar content and pituitary stalk. A transnasal exploration was initially performed and revealed a lymphocytic hypophysitis. light microscopy showed a dense infiltration of mature lymphocytes and plasma cells in the interstitium of the anterior pituitary gland. The stalk area could not be exposed to exclude a germinoma. One year later the lesion relapsed despite dexamethason therapy and a second operation by another neurosurgeon had to be performed. light microscopy showed lymphocytic infiltrates, fibrosis and necrosis. The diagnosis was a lymphocytic hypophysitis again. Though transcranially exposed only pituitary tissue was removed. No infundibular mass became visible at surgery as shown by MRI. The girl developed five months later multiple cerebral lesions, which revealed to be a germinoma. Lymphocytic hypophysitis in children is very rare and a coincidence with a germinoma has not been described from histopathological aspect until now. The origin of the pituitary infiltration is discussed.
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7/95. Imaging in pituitary tuberculosis.

    tuberculosis of the pituitary gland is extremely uncommon and may masquerade as a nonfunctional pituitary neoplasm. We describe imaging findings in three patients with pituitary tuberculosis and review the pattern of involvement and importance of imaging in its management. An accurate diagnosis was found to be important in these cases as antituberculous chemotherapy was curative.
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8/95. diabetes insipidus and anterior pituitary insufficiency as presenting features of Wegener's granulomatosis.

    Central diabetes insipidus (DI) is a rare complication of Wegener's granulomatosis (WG), which usually presents after pulmonary or kidney involvement. Anterior pituitary dysfunction secondary to WG has been extremely rare, documented in only three cases. We report a case of a 47-year-old postmenopausal woman who was diagnosed with hypopituitarism in November 1999 and started on vasopressin, thyroxine, and hydrocortisone. She sought treatment at the Mayo Clinic in February 2000 with a purpuric rash, fever, cough, shortness of breath, and blood in the sputum. Computed tomography of the chest showed a 6-cm irregular mass in the right lower lobe, and a biopsy of the mass showed marked reactive atypia and necrosis. Positive C-antineutrophil cytoplasmic antibodies (ANCA) and skin biopsy of a purpuric lesion showing leukocytoclastic vasculitis confirmed the diagnosis of WG. Hormonal studies showed low gonadotropins, thyroid-stimulating hormone (TSH), and prolactin. magnetic resonance imaging (MRI) of the head showed cystic enlargement of the pituitary gland that did not enhance with gadolinium. Two months into the treatment with cyclophosphamide and prednisone, she had persistent pituitary dysfunction, despite the normal appearance of the pituitary gland on repeat MRI. We conclude that WG should be included in the differential diagnosis of DI and anterior pituitary dysfunction in the proper clinical setting. early diagnosis and treatment may be crucial in preventing pituitary gland destruction and long-term endocrine sequelae. We suggest screening for anterior pituitary failure in the presence of the WG-associated DI.
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9/95. Lymphoid "hypophysitis" with end organ insufficiency.

    A clinically hypothyroid patient had bi-lateral adrenal cortical atrophy and an extraordinary lesion of the pituitary gland. The parenchyma of the adenohypophysis was extensively replaced by a predominantly lymphocytic infiltrate with formation of nodules, many of which had pale germinal centers. There were areas of hyalinization with interstitial fibrosis and mildcapsular thickening. A similar lesion of the adenohypophysis has been reported previously, but without the evidence of adenohypophyseal insufficiency present in this case. It is speculated that this lesion may be related to cell-mediated autoimmunity and other organ-specific autoimmune disorders.
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10/95. The spectrum and significance of primary hypophysitis.

    Hypophysitis can present clinically as a mass lesion of the sella turcica. Secondary hypophysitis occurs in cases where a definite etiologic agent or process inciting the inflammatory reaction can be identified. In contrast, primary hypophysitis refers to inflammation confined to the pituitary gland with no identifiable etiologic associations. We report three cases of primary hypophysitis to illustrate the spectrum of three clinicopathological entities that encompass this disease: lymphocytic hypophysitis, granulomatous hypophysitis, and xanthomatous hypophysitis. Our three patients underwent surgery, with variable response. However, conservative, supportive treatment with or without surgical decompression is generally favored over aggressive and extensive surgical resection that results in hypopituitarism. We conclude that the optimal management of patients with hyophysitis requires a high index of suspicion before extensive surgical resection. Histological confirmation of the diagnosis of hypophysitis can be obtained by performing a biopsy or by requesting an intraoperative frozen section consultation.
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